絲氨酸棕櫚酰轉(zhuǎn)移酶2抗體規(guī)格 英文名稱  Anti-SPTLC2

中文名稱  絲氨酸棕櫚酰轉(zhuǎn)移酶2抗體規(guī)格 

別    名 Serine Palmitoyltransferase; SPTLC-2; LCB 2; LCB2; LCB2a; Long chain base biosynthesis protein 2; Long chain base biosynthesis protein 2a; Serine palmitoyl CoA transferase 2; Serine palmitoyltransferase 2; Serine palmitoyltransferase long chain base subunit 2; Serine palmitoyltransferase subunit II; Serine-palmitoyl-CoA transferase 2; SPT 2; SPT2; SPTC2_HUMAN; SPTLC 2; Sptlc2.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.2ml/200μg

抗體來源  Rabbit

克隆類型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat, Dog, Pig, Cow, Horse

產(chǎn)品類型   一抗  

研究領(lǐng)域     心 細(xì)胞生物 信號(hào)轉(zhuǎn)導(dǎo)

蛋白分子量  predicted molecular weight: 62kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human SPTLC2

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

絲氨酸棕櫚酰轉(zhuǎn)移酶2抗體規(guī)格 產(chǎn)品應(yīng)用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:20-100 IF=1:50-200

（石蠟切片需做抗原修復(fù)） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存條件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

產(chǎn)品介紹 This gene encodes a long chain base subunit of serine palmitoyltransferase. Serine palmitoyltransferase, which consists of two different subunits, is the key enzyme in sphingolipid biosynthesis. It catalyzes the pyridoxal-5-prime-phosphate-dependent condensation of L-serine and palmitoyl-CoA to 3-oxosphinganine. Mutations in this gene were identified in patients with hereditary sensory neuropathy type I. [provided by RefSeq, Mar 2011].

Function : Serine palmitoyltransferase (SPT). The heterodimer formed with LCB1/SPTLC1 constitutes the catalytic core. The composition of the serine palmitoyltransferase (SPT) complex determines the substrate preference. The SPTLC1-SPTLC2-SSSPTA complex shows a strong preference for C16-CoA substrate, while the SPTLC1-SPTLC2-SSSPTB complex displays a preference for C18-CoA substrate.

Subunit : eterodimer with SPTLC1. Component of the serine palmitoyltransferase (SPT) complex, composed of LCB1/SPTLC1, LCB2 (SPTLC2 or SPTLC3) and ssPT (C14orf147/SSSPTA and C3orf57/SSSPTB).

Subcellular Location : Endoplasmic reticulum membrane; Single-pass membrane protein.

Tissue Specificity : Widely expressed.

DISEASE : Defects in SPTLC2 are the cause of hereditary sensory and autonomic neuropathy type 1C (HSAN1C) [MIM:613640]. It is a form of hereditary sensory and autonomic neuropathy, a genetically and clinically heterogeneous group of disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and by prominent sensory abnormalities with a variable degree of motor and autonomic dysfunction. The neurological phenotype is often complicated by severe infections, osteomyelitis, and amputations. HSAN1C symptoms include loss of touch and vibration in the feet, dysesthesia and severe panmodal sensory loss in the upper and lower limbs, distal lower limb sensory loss with ulceration and osteomyelitis, and distal muscle weakness.

Similarity : Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.

Database links :

Entrez Gene: 9517 Human

Entrez Gene: 20773 Mouse

Entrez Gene: SPTLC2 Pig

Entrez Gene: 366697 Rat

Entrez Gene: 553646 Zebrafish

Omim: 605713 Human

SwissProt: O15270 Human

SwissProt: P97363 Mouse

Unigene: 435661 Human

Unigene: 565 Mouse

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